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Von Willebrand’s Disease ( vWD )

Von Willebrand’s Disease ( vWD ). Among women with unexplained menorrhagia. vWD Prevalence. The commonest congenital bleeding disorder at 1 to 2/100 Autosomal inheritance, mostly dominant

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Von Willebrand’s Disease ( vWD )

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  1. Von Willebrand’s Disease (vWD) Among women with unexplained menorrhagia

  2. vWD Prevalence • The commonest congenital bleeding disorder at 1 to 2/100 • Autosomal inheritance, mostly dominant • Multiple different types comprising quantitative or qualitative defects of von Willebrand Factor (vWF) • Considerable heterogeneity in clinical phenotype and in genotype

  3. vWD Clinical Perspective 1 • Commonest signs: epistaxes; menorrhagia; post-operative bleeds; oral and GI bleeding (angiodysplasia) • Both genders affected equally, but menses, pregnancy & childbirth mean women are more vulnerable. Over 60% of vWD patients are women (Cabrera, 1989) • Menorrhagia (>80 ml/cycle) and iron deficiency in >65%. Begins at menarche.

  4. vWD Clinically 2 • Morbidity in a critical sector of society, when demands of family and work are greatest • Lack of awareness of medical community • No objective criteria for estimating blood loss • Cultural barriers exist in Iran, preventing discussion. In affected families, menorrhagia may be considered normal • Pregnancy usually well tolerated

  5. vWD Clinically 3 • Prevalence of vWD in women with menorrhagia is greater than perceived: 11 studies of 998 women with menorrhagia showed 13% had some form of vWD (Kadir, 2004) Average time to diagnosis in US: 16 years due to unawareness. In UK & US, only 2% and 4% of specialists considered vWD screening for menorrhagia Unawareness may lead to early hysterectomy

  6. vWD Clinically 4 • US College of Obst/Gynae (2001) recommended vWD screening for: • Adolescents with severe menorrhagia • Women before hysterectomy for menorrhagia • Adults where no cause for menorrhagia found Kirtava, 2003, reports other gynae. problems in vWD: dysmenorrhea (in 50%); ovarian cyst; endometriosis; even haemoperitoneum

  7. Diagnosis of vWD • Laboratory investigation complex and not widely available: ABO Blood Group; Template Bleeding Time; PFA-100 closure time; Platelet count; FVIII:C; vWF:Ag; vWF: RiCoF; vWF:CB; vWF: FVIIIB; RIPA; HMW Multimer Analysis; Molecular Genetic studies; MCMDM-1vWD Questionnaire (70% sensitive; 98% specific)

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